Hydrocephalus – Causes, Symptom and Surgical Treatments (2023)

Definition

Hydrocephalus is a condition in which excess cerebrospinal fluid (CSF) builds up within the fluid-containing cavities or ventricles of the brain. The term hydrocephalus is derived from the Greek words "hydro" meaning water and "cephalus" meaning the head. Although it translates as "water on the brain," the word actually refers to the buildup of cerebrospinal fluid, a clear organic liquid that surrounds the brain and spinal cord. CSF is in constant circulation within the ventricles of the brain and serves many crucial functions: 1) it acts as a "shock absorber" for the brain and spinal cord; 2) it acts as a vehicle for delivering nutrients to the brain and removing waste from it; and 3) it flows between the cranium and spine to regulate changes in pressure.

When CSF builds up around the brain, it can create harmful pressures on the tissues of the brain confined within the skull. The accumulation of CSF occurs due to either an increase in production of the fluid, a decrease in its rate of absorption or from a condition that blocks its normal flow through the ventricular system.

Hydrocephalus can occur at any age, but is most common in infants and adults age 60 and older. According to the National Institute of Neurological Disorders and Stroke (NINDS), hydrocephalus is believed to affect approximately one to two in every 1,000 children born in the U.S. The majority of these cases are often diagnosed before birth, at the time of delivery or in early childhood.

Causes

Little is understood about the causes of hydrocephalus. Some cases of hydrocephalus are present at birth, while others develop in childhood or adulthood. Hydrocephalus can be inherited genetically, may be associated with developmental disorders, like spina bifida or encephalocele, or occur as a result of brain tumors, head injuries, hemorrhage or diseases such as meningitis. Based on onset, presence of structural defects or high vs. normal CSF pressures, hydrocephalus can be divided into categories.

Acquired Hydrocephalus: This is the type of hydrocephalus that develops at birth or in adulthood and is typically caused by injury or disease.

(Video) Hydrocephalus, Causes, Signs and Symptoms, Diagnosis and Treatment.

Congenital Hydrocephalus: It is present at birth and may be caused by events that occur during fetal development or as a result of genetic abnormalities.

Communicating Hydrocephalus: This type of hydrocephalus occurs when there is no obstruction to the flow of CSF within the ventricular system. The condition arises either due to inadequate absorption or due to an abnormal increase in the quantity of CSF produced.

Non-communication (Obstructive) Hydrocephalus: It occurs when the flow of CSF is blocked along one of more of the passages connecting the ventricles, causing enlargement of the pathways upstream of the block and leading to an increase in pressure within the skull.

Normal Pressure Hydrocephalus: It is a form of communicating hydrocephalus that can occur at any age, but is most common in the elderly. It is characterized by dilated ventricles with normal pressure within the spinal column.

Hydrocephalus Ex-vacuo: It primarily affects adults and occurs when a degenerative disease, like Alzheimer’s disease, stroke or trauma, causes damage to the brain that may cause the brain tissue to shrink.

Symptoms

The symptoms of hydrocephalus tend to vary greatly from person to person and across different age groups. Infants and young children are more susceptible to symptoms from increased intracranial pressure like vomiting and adults can experience loss of function like walking or thinking.

Infants

(Video) Hydrocephalus - Causes, Types, Signs and Symptoms, Pathophysiology, Diagnosis and Treatment

  • Unusually large head size
  • Rapidly increasing head circumference
  • Bulging and tense fontanelle or soft spot
  • Prominent scalp veins
  • Downward deviation of eyes or sunset sign
  • Vomiting
  • Sleepiness
  • Irritability
  • Seizures

Children and Adolescents

  • Nausea and vomiting
  • Swelling of the optic disc or papilledema
  • Blurred or double vision
  • Balance and gait abnormalities
  • Slowing or loss of developmental progress
  • Changes in personality
  • Inability to concentrate
  • Seizures
  • Poor appetite
  • Urinary incontinence

Adults

  • Headache
  • Nausea and vomiting
  • Difficulty walking or gait disturbances
  • Loss of balance or coordination
  • Lethargy
  • Bladder incontinence
  • Impaired vision
  • Impaired cognitive skills
  • Memory loss
  • Mild dementia

Testing and Diagnosis

Once a physician suspects hydrocephalus, he/she performs a thorough clinical evaluation, including reviewing and recording a detailed patient history and performing a physical exam to assess the condition. A complete neurological examination, including one of more of the following tests, is usually recommended to confirm the diagnosis and assess for treatment options:

The tests may reveal useful information about the severity of the condition and its likely cause. Once hydrocephalus is suspected, it is important that a neurosurgeon and/or neurologist become part of the medical team for their expertise on interpreting test results and treating the condition.

(Video) What is Hydrocephalus and Why Does Fluid Build Up in the Brain?

Treatment

Hydrocephalus can be treated in a variety of ways. Based on the underlying etiology, the condition may be treated directly by removing the cause of CSF obstruction or indirectly by diverting the excess fluid. Hydrocephalus is most commonly treated indirectly by implanting a device known as a “shunt” to divert the excess CSF away from the brain. The shunt is a flexible tube which, along with a catheter and a valve, is placed under the skin to drain excess CSF from a ventricle inside the brain to another body cavity such as the peritoneal cavity (the area surrounding the abdominal organs).

Once inserted, the shunt system usually remains in place for the duration of a patient's life (although additional operations to revise the shunt system are sometimes needed). The shunt system continuously performs its function of diverting the CSF away from the brain, thereby keeping the intracranial pressure within normal limits. In some cases, two procedures are performed, the first to divert the CSF and another at a later stage to remove the cause of obstruction (e.g. a brain tumor).

A limited number of patients can be treated with an alternative operation called endoscopic third ventriculostomy. In this procedure, a surgeon utilizes a tiny camera (endoscope) with fiber optics to visualize the ventricles and create a new pathway through which CSF can flow.

Follow-up

Neurological function will be evaluated after surgery. If any neurological problems persist, rehabilitation may be required to further improvement. However, recovery may be limited by the extent of the damage already caused by the hydrocephalus and by the brain's ability to heal.

Because hydrocephalus is an ongoing condition, long-term follow-up by a doctor is required. Follow-up diagnostic tests, including CT scans, MRIs and x-rays, are help determine if the shunt is working properly. A physician should be contacted if any of the following postoperative symptoms are experienced:

  • Redness, tenderness, pain or swelling of the skin along the length of the tube or incision
  • Irritability or drowsiness
  • Nausea, vomiting, headache or double vision
  • Fever
  • Abdominal pain
  • Return of preoperative neurological symptoms

Prognosis

The prognosis for hydrocephalus depends on the cause, the extent of symptoms and the timeliness of diagnosis and treatment. Some patients show a dramatic improvement with treatment, while others do not. In some instances of normal pressure hydrocephalus, dementia can be reversed by shunt placement. Other symptoms, such as headaches, may disappear almost immediately if the symptoms are related to elevated pressure.

(Video) Hydrocephalus and its treatment | Boston Children’s Hospital

In general, the earlier hydrocephalus is diagnosed, the better the chance for successful treatment. The longer the symptoms have been present, the less likely it is that treatment will be successful. Unfortunately, there is no way to accurately predict how successful surgery will be for each individual. Some patients will improve dramatically, while others will reach a plateau or decline after a few months.

Shunt malfunction or failure may occur. The valve can become clogged or the pressure in the shunt may not match the needs of the patient, requiring additional surgery. In the event of an infection, antibiotic therapy may be needed and likely temporary removal of the shunt and replacement by a drain until the infection clears. The shunt can then be re-implanted. A shunt malfunction may be indicated by headaches, vision problems, irritability, fatigue, personality change, loss of coordination, difficulty in waking up or staying awake, a return of walking difficulties, mild dementia or incontinence. In infants, the symptoms of shunt malfunction can include the above as well as vomiting, inappropriate head growth and/or sunsetting eyes. When a shunt malfunctions, surgery is often needed to replace the blocked or malfunctioning portion of the shunt system. Fortunately, most complications can be dealt with successfully.

Author Information

Patient Pages are authored by neurosurgical professionals, with the goal of providing useful information to the public.

Lawrence M. Shuer, MD, FAANS
Rut Thakkar

The AANS does not endorse any treatments, procedures, products or physicians referenced in these patient fact sheets. This information is provided as an educational service and is not intended to serve as medical advice. Anyone seeking specific neurosurgical advice or assistance should consult his or her neurosurgeon, or locate one in your area through the AANS’ Find a Board-certified Neurosurgeon” online tool.

FAQs

What is the surgery for hydrocephalus? ›

The most common treatment for hydrocephalus is the surgical insertion of a drainage system, called a shunt. It consists of a long, flexible tube with a valve that keeps fluid from the brain flowing in the right direction and at the proper rate. One end of the tubing is usually placed in one of the brain's ventricles.

What is the typical cause of hydrocephalus? ›

Normal Pressure Hydrocephalus (NPH) can be the result of bleeding in the brain's CSF (subarachnoid or intraventricular hemorrhage), head trauma, infection, tumor, or a complication of surgery. However, many people develop NPH when none of these factors are present.

What are the symptoms of hydrocephalus in adults? ›

Symptoms of Adult-onset Hydrocephalus
  • Headaches.
  • Nausea.
  • Difficulty focusing the eyes.
  • Unsteady walk or gait.
  • Leg weakness.
  • Sudden falls.
  • Irritability.
  • Drowsiness.

What are 4 types of hydrocephalus? ›

The four main types of hydrocephalus are communicating hydrocephalus, non-communicating hydrocephalus, normal pressure hydrocephalus and hydrocephalus ex-vacuo. Communicating hydrocephalus occurs when the flow of CSF is blocked after it leaves your ventricles.

What are the problems of hydrocephalus? ›

Normal pressure hydrocephalus (NPH) is an uncommon and poorly understood condition that most often affects people over the age of 60. It can sometimes develop after an injury or a stroke, but in most cases the cause is unknown. Mobility problems, dementia and urinary incontinence are the main symptoms of NPH.

What is the latest treatment for hydrocephalus? ›

The most common treatment for hydrocephalus is a medical device called a shunt, a flexible tube, which is placed in the ventricular system of the brain that diverts the flow of CSF to another region of the body, most often the abdominal cavity, or heart, where it can be absorbed.

How long is hydrocephalus surgery? ›

The operation

Shunt surgery is done by a specialist in brain and nervous system surgery (neurosurgeon). It's done under a general anaesthetic and usually takes 1 to 2 hours. You may need to stay in hospital for a few days after the operation to recover.

How successful is hydrocephalus surgery? ›

Idiopathic normal pressure hydrocephalus (iNPH) is a treatable gait disorder and one of very few treatable causes of dementia, most often also causing balance and urinary disturbances [25]. Treatment by shunt surgery is effective with substantial clinical improvement in up to 80% of the patients [4, 25, 43].

What drugs can cause hydrocephalus? ›

A form of toxic leukoencephalopathy causing cerebellar oedema and hydrocephalus has also been described resulting from inhalation of heroin, and also from methadone poisoning [1, 3].

Can hydrocephalus cause death? ›

Left untreated, hydrocephalus can cause severe disability, even death. Hydrocephalus occurs when excessive cerebrospinal fluid accumulates in the brain.

What is the prevention of hydrocephalus? ›

You can't prevent hydrocephalus, but you can lower your risk and your child's risk for developing the condition in the following ways: Prenatal care. Make sure you get prenatal care during pregnancy. This can help reduce your chance of going into premature labor, which can lead to hydrocephalus.

Does hydrocephalus affect memory? ›

The major cognitive impairment in patients with idiopathic normal pressure hydrocephalus (iNPH) is frontal lobe symptoms, such as psychomotor slowing and impairment of attention, working memory, verbal fluency and executive function.

How long do hydrocephalus patients live? ›

What is the life expectancy of a child who has hydrocephalus? Children often have a full life span if hydrocephalus is caught early and treated. Infants who undergo surgical treatment to reduce the excess fluid in the brain and survive to age 1 will not have a shortened life expectancy due to hydrocephalus.

How is hydrocephalus detected? ›

CT scans and MRI scans are often used in combination to confirm a diagnosis of hydrocephalus present from birth (congenital) and hydrocephalus that develops later in children and adults (acquired). These scan the brain in detail.

What is the most common cause of hydrocephalus in adults? ›

Hydrocephalus that develops in children and adults (acquired hydrocephalus) is usually the result of an injury or illness. Possible causes of acquired hydrocephalus include: bleeding inside the brain – for example, if blood leaks over the surface of the brain (subarachnoid haemorrhage)

Which is a risk factor for having hydrocephalus? ›

Consanguinity, maternal diabetes, pregnancy-induced hypertension, oligohydramnios, family history of hydrocephalus, drugs intake during pregnancy, lack of prenatal care, multiparous gestation and alcohol use during pregnancy are some reported risk factors associated with the occurrence of CHC.

What is the most common type of hydrocephalus? ›

Normal pressure hydrocephalus (NPH) is a form of communicating hydrocephalus that can strike people at any age, but it is most common among the elderly. It may result from a subarachnoid hemorrhage, head trauma, infection, tumor or complications of surgery.

Is shunt surgery safe? ›

VP shunts are generally safe, but there are some risks during and after the surgery. There can be bleeding, or an infection can develop.

How is hydrocephalus treated without surgery? ›

Most patients are managed by shunting using a silicone tube and valve system, where CSF is diverted from the cerebral ventricles to another body site [3]. Treatment by shunting is associated with frequent complications, particularly obstruction and infection in young infants, which add to morbidity and mortality.

Is shunt surgery painful? ›

During VP shunt surgery, the doctor placed two small tubes (catheters) and a valve under your skin. After surgery, your neck or belly may feel tender. You will probably feel tired, but you should not have much pain.

What are the side effects of hydrocephalus surgery? ›

Shunt infection
  • redness and tenderness along the line of the shunt.
  • a high temperature.
  • headache.
  • vomiting.
  • neck stiffness.
  • tummy pain if the shunt drains into your tummy.
  • irritability or sleepiness in babies.

Does hydrocephalus go away after surgery? ›

Recovery from normal pressure hydrocephalus is possible with shunt surgery, but improvement varies from person to person. Walking problems, mild dementia, and bladder control issues usually show some improvement within days after surgery.

How long is the recovery period for brain surgery? ›

How Long Does It Take to Recover After Brain Surgery? In total, it typically takes about 4-8 weeks to make a full recovery from a brain surgery. The initial incisions on your head may be sore for about a week afterwards. You may have some mild headaches for a period of about 4-8 weeks as well.

Can hydrocephalus be cured permanently? ›

There is no cure for hydrocephalus, but with timely diagnosis and treatment, children with hydrocephalus can lead normal lives. They will require close care from physicians, neurosurgeons and sometimes neurologists as well.

How much does it cost to treat hydrocephalus? ›

The average cost for the treatment of hydrocephalus in Hyderabad is INR 6,00,000.

How long are you in the hospital after shunt surgery? ›

The actual surgical procedure to implant a shunt typically requires about an hour in the operating room. Afterward, you will be carefully observed for 24 hours. Your stay in the hospital will generally be for two to four days total.

How is hydrocephalus transmitted? ›

The cause of hydrocephalus is not known. Very few cases are caused by a birth defect; others can follow hemorrhage, viral infection, or meningitis. A genetic predisposition has been proposed, with transmission through autosomal recessive or X-linked genes.

What is the treatment for normal pressure hydrocephalus? ›

Treatment for NPH involves surgical placement of a shunt in the brain to drain excess CSF into the abdomen where it can be absorbed as part of the normal circulatory process. This allows the brain ventricles to return to their normal size.

Can hydrocephalus affect your heart? ›

Chronic adult hydrocephalus may increase the risk of cardiovascular disease via compression of cardioregu- latory nuclei near the hypothalamus, as already described.

Is hydrocephalus genetic? ›

Hydrocephalus can be inherited genetically, may be associated with developmental disorders, like spina bifida or encephalocele, or occur as a result of brain tumors, head injuries, hemorrhage or diseases such as meningitis.

Can hydrocephalus make you blind? ›

Patients with untreated hydrocephalus or those with VPS malfunction have been reported to present with visual field defects and transient or permanent blindness. The blindness may be due to involvement of either the anterior or posterior visual pathways.

Can hydrocephalus be treated naturally? ›

Presently, the medical community has no pharmacological remedy for hydrocephalus, which is treated exclusively by surgical methods. Most commonly, a tube is inserted into the fluid-filled cavities of the brain and the excessive liquid is drained to elsewhere in the body.

Do people with hydrocephalus sleep a lot? ›

Hydrocephalus and Insomnia

Another of the physical symptoms is also sleepiness, although many have difficulty falling and staying asleep due to headaches or pain. Insomnia and hydrocephalus feed off of each other, as the person finds it difficult to fall asleep due to head pain, irritability or other physical symptoms.

Which nerve is affected in hydrocephalus? ›

Third cranial nerve dysfunction as a predominant sign of hydrocephalus is very rare and may raise doubts as to the real significance of the imaging findings of enlarged ventricles.

Can hydrocephalus affect your eyes? ›

Hydrocephalus can affect vision in two different ways: 1) Damage to Optic Nerve, 2) Damage to Nerves that Control the Eye Muscles. Visual information is transmitted to the brain by the optic nerve, a bundle of fibers that run from the eye to the brain.

Why is hydrocephalus a serious problem? ›

Hydrocephalus is the buildup of too much cerebrospinal fluid in the brain. Normally, this fluid cushions your brain. When you have too much, though, it puts harmful pressure on your brain. Hydrocephalus can be congenital, or present at birth.

Can I live a normal life with a shunt? ›

Overview. Many people with normal pressure hydrocephalus enjoy a normal life with the help of a shunt. Regular, ongoing checkups with the neurosurgeon will help ensure that your shunt is working correctly, your progress is on track, and you are free to keep living the way you want.

Is brain damage from hydrocephalus reversible? ›

Normal pressure hydrocephalus is one of the few causes of dementia that can be controlled or reversed with treatment.

How does a neurologist diagnose hydrocephalus? ›

Diagnosis of Hydrocephalus

A diagnosis is made by using brain imaging techniques such as ultrasonography, computed tomography (CT), magnetic resonance imaging (MRI), and a thorough neurological evaluation during a doctor visit. More tests are often performed in adults in order to diagnose the condition.

What foods help with hydrocephalus? ›

The diet is characterised by:
  • high consumption of extra virgin olive oil rather than other fats.
  • high intake of fish.
  • high intake of fruit, vegetables, cereals and legumes.
  • moderate intake of alcohol (usually red wine)
  • low intake of meat (in particular, red meat)
  • low to moderate intake of dairy products.

At what age is hydrocephalus detected? ›

Hydrocephalus is typically detected through a prenatal ultrasound between 15 and 35 weeks gestation. Our specialists are able to confirm this diagnosis with a fetal magnetic resonance imaging (MRI) exam, which provides more detailed images of the brain.

When can you detect hydrocephalus? ›

Hydrocephalus is typically detected through a prenatal ultrasound between 15 and 35 weeks gestation. Our specialists are able to confirm this diagnosis with a fetal magnetic resonance imaging (MRI) exam, which provides more detailed images of the brain.

When does hydrocephalus appear? ›

When a baby is born with hydrocephalus, it doesn't usually develop until the third trimester of the mother's pregnancy. Fetal ultrasound is used to diagnose hydrocephalus when a baby is still in the womb. In infants and older children, hydrocephalus is diagnosed with one or more of the following tests: ultrasound.

Can you have hydrocephalus and not know it? ›

Hydrocephalus that is present at birth is referred to as congenital hydrocephalus. Sometimes it does not cause symptoms until adolescence or adulthood even though it is a condition that existed at birth. It is unclear why hydrocephalus can remain dormant for many years only to cause symptoms later in life.

Can you see hydrocephalus on MRI? ›

Congenital and acquired hydrocephalus

CT scans and MRI scans are often used in combination to confirm a diagnosis of hydrocephalus present from birth (congenital) and hydrocephalus that develops later in children and adults (acquired). These scan the brain in detail.

What type of hydrocephalus is most common? ›

Normal pressure hydrocephalus (NPH) is a form of communicating hydrocephalus that can strike people at any age, but it is most common among the elderly.

Where is hydrocephalus located? ›

Hydrocephalus is a condition in which excess cerebrospinal fluid (CSF) builds up within the fluid-containing cavities or ventricles of the brain. The term hydrocephalus is derived from the Greek words "hydro" meaning water and "cephalus" meaning the head.

What part of the brain does hydrocephalus affect? ›

Hydrocephalus, also called water in the brain, is a condition where there is an abnormal build up of CSF (cerebrospinal fluid) in the cavities (ventricles) of the brain. The build-up is often caused by an obstruction that prevents proper fluid drainage.

How long is recovery from hydrocephalus? ›

Recovery Time After Shunt Surgery

It is not uncommon to return back to work or school within a week but this depends on whether your job is sedentary or active. It is advised to avoid heavy lifting, intense exercise, and contact sports for 4–6 weeks while recovering from hydrocephalus surgery.

Can you cure hydrocephalus without surgery? ›

Hydrocephalus is a condition in which cerebrospinal fluid (CSF) abnormally accumulates in the brain. There is currently no prevention or cure, but it can be managed with surgery.

How serious is brain shunt surgery? ›

A shunt is permanent, but because it can malfunction, it may have to be repaired or replaced throughout a person's life. Other rare but serious problems can include infection and bleeding, usually within the first few weeks after the surgery.

Videos

1. Hydrocephalus Recognition and Treatment Video – Brigham and Women’s Hospital
(Brigham And Women's Hospital)
2. Hydrocephalus: Nursing Process
(Osmosis)
3. 2-Minute Neuroscience: Hydrocephalus
(Neuroscientifically Challenged)
4. Normal Pressure Hydrocephalus | Patricia's Story
(Johns Hopkins Medicine)
5. Water on the Brain (Hydrocephalus) : Causes, Diagnosis, Symptoms, Treatment, Prognosis
(Dr. Warraich Health & Food Channel)
6. Normal Pressure Hydrocephalus: Dr. Adam Mednick describes NPH symptoms & treatment.
(Dr. Adam Mednick)
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